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Long-term Follow-up of Hb Madrid Patient / 임상소아혈액종양
Clinical Pediatric Hematology-Oncology ; : 55-58, 2013.
Artigo em Coreano | WPRIM | ID: wpr-788482
ABSTRACT
We report a long-term follow-up of unstable hemoglobin (Hb) patient. He was diagnosed as Hb Madrid [beta115(G17)Ala-->Pro] by direct DNA sequencing and restriction enzyme analysis. Hydroxyurea had been given for beta-chain hemoglobinopathies through activation of gamma(gamma)-chain synthesis. Nowadays he still needs transfusion three or four times per year, but he had been free of hemolytic crisis after hydroxyurea. Although he has been treated for hemochromatosis with parenteral and oral iron-chelating agents, liver cirrhosis complicated by esophageal varix was developed and treated with endoscopic ligation. In addition, he is on warfarin maintenance for anticoagulation therapy for extensive portal vein and superior mesenteric vein thrombosis which presented with abdominal pain and diagnosed by CT scan. In management of unstable Hb patients, physician should monitor and control the serum ferritin level with iron-chelating agents, and be aware of possible long-term complication including hemochromatosis, cirrhosis or thromboembolism.
Assuntos

Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Compostos Organotiofosforados / Veia Porta / Tromboembolia / Trombose / Varfarina / Fibrose / Hemoglobinas / Hemoglobinas Anormais / Mapeamento por Restrição / Varizes Esofágicas e Gástricas Tipo de estudo: Estudo observacional / Estudo prognóstico Limite: Humanos Idioma: Coreano Revista: Clinical Pediatric Hematology-Oncology Ano de publicação: 2013 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Compostos Organotiofosforados / Veia Porta / Tromboembolia / Trombose / Varfarina / Fibrose / Hemoglobinas / Hemoglobinas Anormais / Mapeamento por Restrição / Varizes Esofágicas e Gástricas Tipo de estudo: Estudo observacional / Estudo prognóstico Limite: Humanos Idioma: Coreano Revista: Clinical Pediatric Hematology-Oncology Ano de publicação: 2013 Tipo de documento: Artigo